Sphenoid greater wing dysplasia associated with neurofibromatosis type Ⅰ: a case report and review of the literature
10.3760/cma.j.issn.1009-4598.2018.01.015
- VernacularTitle: 神经纤维瘤病Ⅰ型合并蝶骨大翼发育不良一例报告并文献复习
- Author:
Hansong SHENG
1
;
Maode WANG
;
Jian LIN
;
Fengchun LIN
;
Dandong LI
;
Junhao FANG
;
Nu ZHANG
Author Information
1. Department of Neurosurgery, Second Affiliated Hospital of Wenzhou Medical University, Wenzhou 325027, China
- Publication Type:Review
- Keywords:
Neurofibromatosis type Ⅰ;
Sphenoid wing dysplasia;
Titanium;
Exophthalmos;
Skull base Reconstruction
- From:
Chinese Journal of Plastic Surgery
2018;34(1):64-67
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To discuss the etiology, pathogenesis, clinical manifestation, diagnosis and therapy of sphenoid wing dysplasia(SWD) associated with neurofibromatosis type Ⅰ(NF-Ⅰ).
Methods:We retrospectively reviewed its clinical manifestations, imaging, surgical treatment, complications and postoperative outcome of one NF-Ⅰ patient with SWD.
Results:A 14 years-old girl presented with pulsating exophthalmos, loss of vision and café au lait spots. Radiological studies showed right-side orbital enlargement and complete absence of the greater wing of the sphenoid. Titanium mesh was tailored intraoperatively to close the defect as a barrier between the orbital cavity and the cranium and then covered by periosteum.The patient developed postoperative infectious which was controlled by after antibiotic treatment and proper drainage. Proptosis improved significantly after surgery within a month. Ocular pulsation subsided and clinical symptoms improved at 28-month follow-up.
Conclusions:Sphenoid greater wing dysplasia associated with neurofibromatosis type Ⅰ is a rare inherited autosomal dominant disorders. The treatment should be customized to each patient. Titanium mesh reconstruction is patients with symptomatic sphenoid dysplasia. It can correct the proptosis and pulsating exophthalmos without the risk of bone resorption and recurrence.However, high risk of infection is associated with the procedure.
